Diagnosis and management of acquired von Willebrand syndrome.

Augusto B. Federici, Pier Mannuccio Mannucci

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

The acquired von Willebrand syndrome is a rare bleeding disorder with laboratory finding similar to those of congenital von Willebrand's disease. Unlike the congenital form, however, the acquired syndrome usually occurs in persons with no personal or family history of bleeding. Large-scale studies are not available, diagnosis is still difficult, and treatment is empirical. Published findings and an international registry indicate that the syndrome is especially frequent in lympho- or myeloproliferative disorders; therefore, it should be suspected when there is excessive bleeding in patients with these disorders. Acquired von Willebrand syndrome is also associated with solid tumors, immunologic and cardiovascular, disorders. Diagnosis is based on assays measuring ristocetin cofactor activity or collagen binding; these levels are usually abnormally low, while factor VIII coagulant activity can be normal. Factor VIII/von Willebrand factor-inhibiting activities are found in only a minority of cases. Bleeding episodes in patients with the syndrome are mostly of the mucocutaneous type and can be managed with desmopressin, plasma-derived factor VIII/von Willebrand factor concentrates, and intravenous immunoglobulin. Recombinant activated factor VII, plasmapheresis, corticosteroids, and immunosuppressors combined with chemotherapy are also useful in some cases. Acquired von Willebrand syndrome, although rare, warrants further understanding for clinical practice.

Original languageEnglish
Pages (from-to)169-175
Number of pages7
JournalClinical Advances in Hematology and Oncology
Volume1
Issue number3
Publication statusPublished - Mar 2003

Fingerprint

Hemorrhage
von Willebrand Diseases
Deamino Arginine Vasopressin
Factor VIIa
Myeloproliferative Disorders
Coagulants
Plasmapheresis
Lymphoproliferative Disorders
Intravenous Immunoglobulins
Factor VIII
von Willebrand Factor
Registries
Adrenal Cortex Hormones
Collagen
Drug Therapy
Neoplasms
factor VIII, von Willebrand factor drug combination
Therapeutics

ASJC Scopus subject areas

  • Oncology
  • Hematology

Cite this

Diagnosis and management of acquired von Willebrand syndrome. / Federici, Augusto B.; Mannucci, Pier Mannuccio.

In: Clinical Advances in Hematology and Oncology, Vol. 1, No. 3, 03.2003, p. 169-175.

Research output: Contribution to journalArticle

@article{05267b6d0afc4548b459945c993e70e8,
title = "Diagnosis and management of acquired von Willebrand syndrome.",
abstract = "The acquired von Willebrand syndrome is a rare bleeding disorder with laboratory finding similar to those of congenital von Willebrand's disease. Unlike the congenital form, however, the acquired syndrome usually occurs in persons with no personal or family history of bleeding. Large-scale studies are not available, diagnosis is still difficult, and treatment is empirical. Published findings and an international registry indicate that the syndrome is especially frequent in lympho- or myeloproliferative disorders; therefore, it should be suspected when there is excessive bleeding in patients with these disorders. Acquired von Willebrand syndrome is also associated with solid tumors, immunologic and cardiovascular, disorders. Diagnosis is based on assays measuring ristocetin cofactor activity or collagen binding; these levels are usually abnormally low, while factor VIII coagulant activity can be normal. Factor VIII/von Willebrand factor-inhibiting activities are found in only a minority of cases. Bleeding episodes in patients with the syndrome are mostly of the mucocutaneous type and can be managed with desmopressin, plasma-derived factor VIII/von Willebrand factor concentrates, and intravenous immunoglobulin. Recombinant activated factor VII, plasmapheresis, corticosteroids, and immunosuppressors combined with chemotherapy are also useful in some cases. Acquired von Willebrand syndrome, although rare, warrants further understanding for clinical practice.",
author = "Federici, {Augusto B.} and Mannucci, {Pier Mannuccio}",
year = "2003",
month = "3",
language = "English",
volume = "1",
pages = "169--175",
journal = "Clinical Advances in Hematology and Oncology",
issn = "1543-0790",
publisher = "Millennium Medical Publishing, Inc.",
number = "3",

}

TY - JOUR

T1 - Diagnosis and management of acquired von Willebrand syndrome.

AU - Federici, Augusto B.

AU - Mannucci, Pier Mannuccio

PY - 2003/3

Y1 - 2003/3

N2 - The acquired von Willebrand syndrome is a rare bleeding disorder with laboratory finding similar to those of congenital von Willebrand's disease. Unlike the congenital form, however, the acquired syndrome usually occurs in persons with no personal or family history of bleeding. Large-scale studies are not available, diagnosis is still difficult, and treatment is empirical. Published findings and an international registry indicate that the syndrome is especially frequent in lympho- or myeloproliferative disorders; therefore, it should be suspected when there is excessive bleeding in patients with these disorders. Acquired von Willebrand syndrome is also associated with solid tumors, immunologic and cardiovascular, disorders. Diagnosis is based on assays measuring ristocetin cofactor activity or collagen binding; these levels are usually abnormally low, while factor VIII coagulant activity can be normal. Factor VIII/von Willebrand factor-inhibiting activities are found in only a minority of cases. Bleeding episodes in patients with the syndrome are mostly of the mucocutaneous type and can be managed with desmopressin, plasma-derived factor VIII/von Willebrand factor concentrates, and intravenous immunoglobulin. Recombinant activated factor VII, plasmapheresis, corticosteroids, and immunosuppressors combined with chemotherapy are also useful in some cases. Acquired von Willebrand syndrome, although rare, warrants further understanding for clinical practice.

AB - The acquired von Willebrand syndrome is a rare bleeding disorder with laboratory finding similar to those of congenital von Willebrand's disease. Unlike the congenital form, however, the acquired syndrome usually occurs in persons with no personal or family history of bleeding. Large-scale studies are not available, diagnosis is still difficult, and treatment is empirical. Published findings and an international registry indicate that the syndrome is especially frequent in lympho- or myeloproliferative disorders; therefore, it should be suspected when there is excessive bleeding in patients with these disorders. Acquired von Willebrand syndrome is also associated with solid tumors, immunologic and cardiovascular, disorders. Diagnosis is based on assays measuring ristocetin cofactor activity or collagen binding; these levels are usually abnormally low, while factor VIII coagulant activity can be normal. Factor VIII/von Willebrand factor-inhibiting activities are found in only a minority of cases. Bleeding episodes in patients with the syndrome are mostly of the mucocutaneous type and can be managed with desmopressin, plasma-derived factor VIII/von Willebrand factor concentrates, and intravenous immunoglobulin. Recombinant activated factor VII, plasmapheresis, corticosteroids, and immunosuppressors combined with chemotherapy are also useful in some cases. Acquired von Willebrand syndrome, although rare, warrants further understanding for clinical practice.

UR - http://www.scopus.com/inward/record.url?scp=33644614784&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33644614784&partnerID=8YFLogxK

M3 - Article

VL - 1

SP - 169

EP - 175

JO - Clinical Advances in Hematology and Oncology

JF - Clinical Advances in Hematology and Oncology

SN - 1543-0790

IS - 3

ER -